Background: Paediatric onset Crohn’s disease (CD) is a heterogeneous disorder which is subjected to progression and complications in a substantial proportion of patients.
Aim: We aimed to assess the progression and changes in paediatric CD phenotype on long term follow up.
Methods: Using the Schneider Paediatric Inflammatory Bowel Disease (SPID) cohort, encompassing 482 paediatric onset CD patients seen between 1981 and 2013, we retrospectively evaluated the medical records of 215 patients with at least 10 years of follow-up. Disease phenotype was determined at diagnosis and during follow up at different time points. Phenotype was determined according to the Paris classification. The impact of possible predictors on phenotype progression including age at diagnosis, gender, clinical manifestations, disease location and behaviour was assessed as well as the association between different therapeutic regimens during disease course and phenotype progression.
Results: Progression of disease location, behaviour, and perianal involvement was observed in 28%, 38.2% and 20.4% of patients, respectively, after a median follow-up of 16.4 (± 4.4) years. Microscopic ileocolonic disease at diagnosis and treatment with immunomodulators during the first year following diagnosis were significant predictors for progression of disease extent. Treatment with anti tumor necrosis factor-ɑ agents and number of flares per years of follow-up were associated with progression of disease extent, behaviour and perianal involvement.
Conclusion: Disease`s extent, behaviour and prevalence of perianal disease change significantly over time in paediatric-onset CD. In our cohort, most clinical, laboratory and endoscopic parameters do not serve as predictors for long-term disease progression
